Vasculitis is group of diseases where inflammation of blood vessels is the common feature. Patients typically present with fever, fatigue, weakness and muscle and joint aches. These symptoms are very common among many different diseases, not just vasculitis. A clustering of other symptoms, physical examination findings, blood tests, radiology and biopsy help make the diagnosis. There are currently no criteria to help doctors make a diagnosis of vasculitis when a patient presents with these non specific symptoms and they are reliant on previous experience and disease definitions. One of the aims of this project is to develop diagnostic criteria for the primary systemic vasculitides (granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, Churg Strauss syndrome, polyarteritis nodosa, giant cell arteritis, Takayasu arteritis). We, the investigators, will do this by studying a large group of patients with vasculitis and comparing them to a large group of patients that present in a similar way, but do not have vasculitis. By comparing the 2 groups we will create a list of items to differentiate between vasculitis and 'vasculitis mimics'.

We also aim to update the current classification criteria. Classification criteria are used to group patients into different types of vasculitis, once a diagnosis of vasculitis has been made, and are useful for studying patients in clinical trials with similar or identical diseases. The current classification criteria (American college of Rheumatology 1990 criteria) were developed 20 years ago, before the availability of some important diagnostic tests (e.g. antineutrophil cytoplasmic antibodies [ANCA]), and are now not consistent with some of the current disease definitions. Therefore to progress future research in vasculitis, it is important that the classification criteria are updated. We will recruit 260 patients with each of the 6 types of vasculitis and compare them with 1300 controls (patients with the 5 other types of vasculitis), in order to determine the optimal combination of symptoms, signs and investigations that classify each person into the appropriate group.

Develop new diagnostic and classification criteria for ANCA associated vasculitis and polyarteritis nodosa [ Time Frame: 3 years ] [ Designated as safety issue: No ]

Biospecimen Retention: Samples With DNA

Patients may be given the option of having blood, urine or other tissue obtained as part of their routine care retained for use in future ethically approved studies. They may also be asked to provide additional blood to be stored for this purpose. This component is an optional extra, and does not form part of the main study.

Patients with Wegener's granulomatosis. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Classification criteria.

MPA classification

Patients with microscopic polyangiitis. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Classification criteria.

CSS classification

Patients with Churg Strauss syndrome. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Classification criteria.

PAN classification

Patients with polyarteritis nodosa. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Classification criteria.

Control Classification

For each of the diseases being evaluated (WG, MPA, CSS, PAN, GCA, TAK), patients with the other 5 diseases will be the control group. Within these groups, 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Classification criteria.

WG diagnostic

Patients with a new presentation of Wegener's granulomatosis. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Diagnostic criteria.

MPA diagnostic

Patients with a new presentation of microscopic polyangiitis. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Diagnostic criteria.

CSS diagnostic

Patients with a new presentation of Churg Strauss syndrome. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Diagnostic criteria.

PAN diagnostic

Patients with a new presentation of polyarteritis nodosa. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Diagnostic criteria.

Control diagnostic

Patients without vasculitis, but presenting with similar features to the 6 different types of vasculitis being studied. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Diagnostic criteria.

GCA classification

Patients with giant cell arteritis. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Classification criteria.

TAK classification

Patients with Takayasu arteritis. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Classification criteria.

GCA diagnostic

Patients with a new diagnosis of giant cell arteritis. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Diagnostic criteria.

TAK diagnostic

Patients with a new diagnosis of Takayasu arteritis. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Diagnostic criteria.

Patient or next of kin unable or unwilling to provide informed consent or assent.

Contacts and Locations

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For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT01066208